Intrinsically Disordered Proteins in Human Diseases: Introducing the D2 Concept

VN Uversky, CJ Oldfield, AK Dunker - Annu. Rev. Biophys., 2008 - annualreviews.org
Intrinsically disordered proteins (IDPs) lack stable tertiary and/or secondary structures under
physiological conditions in vitro. They are highly abundant in nature and their functional …

Prion diseases of humans and animals: their causes and molecular basis

J Collinge - Annual review of neuroscience, 2001 - annualreviews.org
▪ Abstract Prion diseases are transmissible neurodegenerative conditions that include
Creutzfeldt-Jakob disease (CJD) in humans and bovine spongiform encephalopathy (BSE) …

2.7 Å cryo-EM structure of ex vivo RML prion fibrils

SW Manka, W Zhang, A Wenborn, J Betts… - Nature …, 2022 - nature.com
Mammalian prions propagate as distinct strains and are composed of multichain assemblies
of misfolded host-encoded prion protein (PrP). Here, we present a near-atomic resolution …

Prion protein monoclonal antibody (PRN100) therapy for Creutzfeldt–Jakob disease: evaluation of a first-in-human treatment programme

S Mead, A Khalili-Shirazi, C Potter, T Mok… - The Lancet …, 2022 - thelancet.com
Background Human prion diseases, including Creutzfeldt–Jakob disease (CJD), are rapidly
progressive, invariably fatal neurodegenerative conditions with no effective therapies. Their …

Conformational constraints for amyloid fibrillation: the importance of being unfolded

VN Uversky, AL Fink - Biochimica et Biophysica Acta (BBA)-Proteins and …, 2004 - Elsevier
Recent reports give strong support to the idea that amyloid fibril formation and the
subsequent development of protein deposition diseases originate from conformational …

Mammalian prions and their wider relevance in neurodegenerative diseases

J Collinge - Nature, 2016 - nature.com
Prions are notorious protein-only infectious agents that cause invariably fatal brain diseases
following silent incubation periods that can span a lifetime. These diseases can arise …

Peptide fibrillization

IW Hamley - Angewandte Chemie International Edition, 2007 - Wiley Online Library
The fibrillization of peptides is relevant to many diseases based on the deposition of
amyloids. The formation of fibrils is being intensively studied, especially in terms of …

Prediction of aggregation-prone regions in structured proteins

GG Tartaglia, AP Pawar, S Campioni… - Journal of molecular …, 2008 - Elsevier
We present a method for predicting the regions of the sequences of peptides and proteins
that are most important in promoting their aggregation and amyloid formation. The method …

Location and properties of metal-binding sites on the human prion protein

GS Jackson, I Murray, LLP Hosszu… - Proceedings of the …, 2001 - National Acad Sciences
Although a functional role in copper binding has been suggested for the prion protein,
evidence for binding at affinities characteristic of authentic metal-binding proteins has been …

Emerging principles of conformation-based prion inheritance

P Chien, JS Weissman… - Annual review of …, 2004 - annualreviews.org
▪ Abstract The prion hypothesis proposes that proteins can act as infectious agents.
Originally formulated to explain transmissible spongiform encephalopathies (TSEs), the …